Descripción clínica y fisiopatológica de la cefalea numular: serie de casos / Clinical and pathophysiological description of nummular headaches: a case series

INTRODUCCIÓN: La cefalea numular se describió inicialmente como una cefalea primaria infrecuente caracterizada por dolor circunscrito crónico en una pequeña área craneal, de intensidad leve-moderada y que puede presentar exacerbaciones. Desde su descripción inicial se han publicado decenas de casos de cefalea numular que amplían el espectro clínico y fisiopatológico. OBJETIVOS: Revisar y establecer las características clínicas de la cefalea numular, analizar la epidemiología en nuestro medio y aclarar incógnitas fisiopatológicas. PACIENTES Y MÉTODOS: Se estudiaron 83 casos (42 mujeres y 41 varones), que fueron diagnosticados de cefalea numular desde julio de 2003 a julio de 2008, y seguidos hasta 2018 en el Hospital Universitario Fundación Alcorcón. La edad media de inicio fue de 46 años. El tiempo de evolución hasta el diagnóstico fue de siete meses. RESULTADOS: El dolor fue moderado-intenso y opresivo, con exacerbaciones en el 62,5% de los casos. El tamaño medio del área sintomática fue de 2,4 ± 0,7 cm, con una localización parietal (47%), temporal (24%), frontal (12%) u occipital (6%). Presentó un patrón crónico (82%) o episódico (18%). Mostraron síntomas de disfunción sensitiva local 25 pacientes, y otros tres, cambios en la piel. La remisión espontánea se observó en el 36% de los casos, y los fármacos más utilizados fueron los antiinflamatorios no esteroideos, el paracetamol y la gabapentina. La toxina botulínica fue eficaz. CONCLUSIONES: La cefalea numular es un trastorno con características bien definidas y con entidad propia. Nuestros datos indican que su origen está en las estructuras epicraneales. La conjunción de dolor, disfunción sensitiva y alteraciones tróficas indica una forma local de síndrome regional complejo

INTRODUCTION: Nummular headache was initially described as an infrequent primary headache characterised by chronic circumscribed mild-to-moderate pain in a small area of the head that may present with exacerbations. Since its initial description, dozens of cases of nummular headache have been reported which broaden the clinical and pathophysiological spectrum. AIMS. To review and establish the clinical characteristics of nummular headache, to analyse the epidemiology in our setting and to clarify some unanswered pathophysiological issues. PATIENTS AND METHODS: The study involved 83 cases (42 women and 41 men) who were diagnosed with nummular headache from July 2008 and followed up until 2018 at the Fundación Alcorcón Hospital. The mean age of onset was 46 years. The time to diagnosis was seven months. RESULTS: The pain was moderate-intense and oppressive, with exacerbations in 62.5% of cases. The average size of the symptomatic area was 2.4 ± 0.7 cm, with a parietal (47%), temporal (24%), frontal (12%) or occipital (6%) location. It presented a chronic (82%) or episodic (18%) pattern. Symptoms of local sensory dysfunction were observed in 25 patients and three others showed skin alterations. Spontaneous remission was observed in 36% of cases, and the most commonly used drugs were non-steroidal anti-inflammatory drugs, paracetamol and gabapentin. Botulinum toxin was effective. CONCLUSIONS: Nummular headache is a disorder with well-defined characteristics and is considered an entity in its own right. Our data indicate that its origin lies in the epicranial structures. The conjunction of pain, sensory dysfunction and trophic disorders suggests a local form of a complex regional síndrome

HLA-A, -B, -DRB1 allele and haplotype frequencies of 920 cord blood units from Central Chile.

We present human leukocyte antigen (HLA) haplotype and allele/antigenic group frequencies derived from a data set of 920 umbilical cord blood units collected in Central Chile. HLA-A and -B genotypes were typed using sequence specific oligonucleotide probe methods while HLA-DRB1 genotypes were obtained from sequencing-based typing. The most frequent haplotype is A*29~B*44~DRB1*07:01 with an estimated frequency of 2.1%.

Depression and anxiety are not related to nummular headache.

Nummular headache (NH) is a clinical picture characterized by head pain that is exclusively felt in a round, elliptical, or oval area of the head. Although there is evidence supporting an organic origin for NH, some authors question this origin, hypothesizing a potential role for psychological factors. Our aims were to investigate the differences in anxiety and depression between NH patients and healthy controls, and to analyse if these conditions were related to pain parameters in NH patients. The Beck depression inventory (BDI-II) and the trait anxiety scale from state-trait anxiety inventory (STAI) were administered to 26 patients with NH and 34 comparable matched controls. No significant interactions between group (NH patients, controls) in either depression (U = 391; p = 0.443) or anxiety levels (U = 336; p = 0.113) were found. Both groups showed similar scores in the BDI-II (patients: 3.9 +/- 2.9; controls: 3.46 +/- 3.15) and STAI (patients: 17.23 +/- 10.3; controls: 13.5 +/- 7.9). Moreover, neither depression nor anxiety showed association with mean pain intensity, pain intensity in exacerbations, size of pain area, or pain frequency. Our study demonstrated that self-reported depression and anxiety were not related to the presence of NH. Further, longitudinal studies are still needed to elucidate the role of mood state in the course of NH.

Local decrease of pressure pain threshold in nummular headache.

BACKGROUND: Nummular headache (NH) is a primary disorder presenting with localized pain that is circumscribed to a coin-shaped area of the head surface. METHODS: In 12 patients with NH (3 men and 9 women, 21 to 67 years old), we measured the pressure pain threshold (PPT) in several points while they were headache-free. The following cephalic and extracephalic points were explored: the symptomatic cranial area, a symmetrical point on the nonsymptomatic side, and 3 standardized pairs of symmetrical points (anterior part of the temporal muscle, upper trapezius muscle, and distal dorsal part of the second finger). Three consecutive PPT readings were obtained with an algometer on each point, and the repeatability of these measurements was always high (ICC: 0.93 to 0.97). RESULTS: Mean PPT was lower in the symptomatic cranial area than in the contra-lateral symmetrical point (1.8 +/- 0.6 kg/cm(2) vs 2.4 +/- 0.6 kg/cm(2); P < .001), whereas in the remaining reference points PPT was almost equal on both sides. CONCLUSIONS: According to these data, NH seems to be associated with a local increase of pain sensitivity to mechanical stimulation.

Are manual therapies effective in reducing pain from tension-type headache?: a systematic review.

OBJECTIVES: A systematic review was performed to establish whether manual therapies have specific efficacy in reducing pain from tension-type headache (TTH). METHODS: Computerized literature searches were performed in MEDLINE, EMBASE, AMED, MANTIS, CINAHL, PEDro, and Cochrane databases. Papers were included if they described clinical (open noncontrolled studies) or randomized controlled trials in which any form of manual therapy was used for TTH, and if they were published after 1994 in the English language. The methodologic quality of the trials was assessed using the PEDro scale. Levels of scientific evidence, based on the quality and the outcomes of the studies, were established for each manual therapy: strong, moderate, limited, and inconclusive evidence. RESULTS: Only six studies met the inclusion criteria. These trials evaluated different manual therapy modalities: spinal manipulation (three trials), classic massage (one trial), connective tissue manipulation (two trials), soft tissue massage (one trial), Dr. Cyriax's vertebral mobilization (one trial), manual traction (one trial), and CV-4 craniosacral technique (one trial). Methodologic PEDro quality scores ranged from 2 to 8 points out of a theoretical maximum of 10 points (mean=5.8+/-2.1). Analysis of the quality and the outcomes of all trials did not provide rigorous evidence that manual therapies have a positive effect in reducing pain from TTH: spinal manipulative therapy showed inconclusive evidence of effectiveness (level 4), whereas soft tissue techniques showed limited evidence (level 3). CONCLUSIONS: The authors found no rigorous evidence that manual therapies have a positive effect in the evolution of TTH. The most urgent need for further research is to establish the efficacy beyond placebo of the different manual therapies currently applied in patients with TTH.

Cluster headache: orbital hemodynamic changes during Valsalva maneuver.

BACKGROUND: The clinical features of cluster headache (CH) disclose some vascular changes in the symptomatic region, but few instrumental studies have assessed orbital hemodynamics in patients with this disorder. METHODS: Orbital blood flow reactivity elicited by Valsalva maneuver (VM) was studied with ophthalmic artery eco-Doppler in 16 patients (14 men and 2 women; mean age: 41.2) suffering from episodic CH and in 18 healthy controls. Patients were examined twice: first, in a cluster period (between pain attacks), and second, in a remission period. Each time peak-systolic and end-diastolic flow velocities were recorded in both ophthalmic arteries at rest and during all phases of VM. RESULTS: Valsalva phase IV was consistently associated with an increment of blood flow velocities through the ophthalmic arteries. Unlike controls, patients showed an asymmetric vascular reactivity. In the cluster period peak-systolic flow velocity increments were lower on the symptomatic side than on the asymptomatic side (14.1% vs. 34.4%; P < .001), while in remission end-diastolic flow velocity increments were higher in the previously symptomatic orbit (129% vs. 72.9%; P < .05). Vascular reactivity on the asymptomatic side was always similar to that of healthy controls. CONCLUSIONS: In episodic CH, the symptomatic orbit shows an abnormal vascular reactivity. During the cluster period, basal vasodilation and hyperemia could preclude it from admitting a much greater amount of blood at the end of Valsalva. During remission, there might be some latent vascular changes that lead to supersensitive vasodilator responses and/or opening of arteriovenous shunts under certain circumstances such as Valsalva. These phenomena could be relevant in the pathophysiology of CH.

Nummular headache: a prospective series of 14 new cases.

OBJECTIVE: To study the clinical features of nummular headache (NH) and get an approach to its epidemiology. BACKGROUND: NH has been recently described as a primary disorder characterized by head pain exclusively felt in a small rounded area typically 2-6 cm in diameter. METHODS: Through a 1-year period we have studied all patients referred to our neurologic clinic because of head pain exclusively felt in a small-circumscribed area, and not attributed to another disorder. All the patients had normal neurological, analytical, and neuroimaging examinations. All the patients belonged within the same regional care system comprising 220,000 inhabitants. RESULTS: A total of 11 females and 3 males were studied. Based in our hospital series, the incidence was 6.4/100,000/year. The mean age at the onset was 38 years (range: 13-72). Only three patients had another concurrent headache: migraine (n = 2), and trigeminal neuralgia (n = 1) which proved to have an independent course. All the patients reported head pain exclusively felt in either a rounded (n = 12) of 1-6 cm diameter, or an oval area (n = 2) of 5 x 3 cm, and 2 x 3 cm, respectively. Both size and shape of the painful area remained constant since the onset of symptoms. The location of the symptomatic area was mostly parietal (n = 7) or temporal (n = 5), but also frontal (n = 1) and in occiput (n = 1). The background pain was mostly mild-to-moderate, but also moderate-to-severe pain was reported. Exacerbations-either spontaneous or precipitated by combing hair or touching the symptomatic area-were reported by 8 patients. The temporal pattern was chronic-continuous (n = 7) and episodic (n = 7). Ten patients reported a variable combination of sensory disturbance (tenderness, hypoesthesia, hyperalgesia, and allodynia) in the symptomatic area. There were no autonomic accompaniments. Treatment was generally not necessary. When needed, standard oral doses of paracetamol usually sufficed. CONCLUSIONS: NH emerges as a clear-cut clinical picture. It is a noninfrequent primary headache. The particular topography suggests the pain has a probable epicranial source conveyed by, or originated in, one/a few terminal branch(es) of the cutaneous nerves of the scalp.

La púrpura en el niño con especial referencia a la púrpura trombopénica inmune / Purpura in the child with special reference to immune thrombopenic purpura

El manejo del niño trombocitopénico no debe ser un problema difícil si se tienen en cuenta principios básicos de la fisiopatología de las plaquetas junto con el conocimiento de la clínica y de las modalidades terapéuticas disponibles. La observación clínica del paciente así como el uso adecuado del laboratorio, en particular del mielograma, son las bases para un diagnóstico correcto tanto en los casos agudos como en los crónicos. El uso juicioso de las formas de tratamiento disponibles, a saber, corticoides, inmunoglubulina endovenosa y esplenectomía y el conocimiento detallado de sus complicaciones constituyen la base para el éxito en el manejo. Como en tantas otras áreas de la pediatría, más daño se puede hacer por precipitarse a hacer una prueba diagnóstica o comenzar un tratamiento, que por tomar tiempo en delinear un plan de evaluación y terapia adecuadas. Por último, el recurso a la consulta con el hematólogo pediatra, en caso de duda ante el paciente purpúrico, dará al clínico la oportunidad de estar al corriente de los progresos en esta enfermedad así como uno opinión cualificada